Antiepileptics drugs are the mainstay of the management of epilepsy in children. Sodium valproate (VPA) and carbamazepine (CBZ) are widely used medications in childhood epilepsy. Hyperammonemia has been described as a known side effect of valproate therapy. It is known that VPA-associated HA is common among patients who hold genetic mutations of the carbomoyl phosphatase synthase 1 gene (CPS1). Aggravation of self-limited epilepsy with centrotemporal spikes (SLECTS) is a rare side effect of CBZ. Here, we present a child who had CBZ-induced aggravation of rolandic epilepsy and VPA-induced HA encephalopathy in the background of an unrecognised heterozygous gene variant of CPS1. An 8-year-old boy with SLECTS presented with a history of abnormal behaviours and drowsiness. He was apparently well until six years when he developed seizures in favour of rolandic epilepsy. His electroencephalogram (EEG) showed bilateral predominantly on the right-sided central-temporal spikes and waves. The diagnosis of SLECTS was made, and he was commenced on CBZ. Though he showed some improvement at the beginning, his seizure frequency increased when the dose of CBZ was increased. His repeat EEG showed electrical status in slow-wave sleep, and CBZ was stopped. Subsequently, he was started on VPA, and with that, he developed features of encephalopathy. He had elevated serum ammonia with normal liver functions. VPA was stopped with the suspicion of VPA-induced hyperammonemia. Tandem mass spectrometry did not show significant abnormality in the amino acid profile. Specific genetic analysis revealed a c.2756 C > T.p (Ser919Leu) heterozygote genetic mutation of the CSP 1 gene. This is a classic example where side effects of treatment determine the choice of antiepileptics drugs (AEDs) in childhood epilepsy. It is essential to keep in mind that SLECTS can be aggravated with certain AEDs, and VPA-induced HA in the absence of live failure could be due to underlying inherited metabolic disorders.
Introduction: Acute asthma is commonly treated with beta agonist therapy in the form of inhaled salbutamol. Clinicians are reluctant to use intravenous (IV) salbutamol in acute management due to the possibility of tachyarrhythmia. Objective: To describe the effects of IV salbutamol on the heart rate and to describe any cardiac rhythm abnormalities or cardiac toxicity (ischaemia) associated with IV salbutamol therapy. Method: A prospective, descriptive, observational study was conducted at the Medical Intensive Care Unit (MICU) Lady Ridgeway Hospital (LRH), Colombo on all children admitted with acute severe asthma from August to December 2015 who required IV salbutamol therapy. Data were collected using a self-administered questionnaire. The Wilcoxon significant rank test was utilized in statistical analysis. Results: During the study period 30 patients were admitted to the MICU for continuous IV salbutamol therapy. The mean heart rate on admission was 197.67±15.61. An 18% reduction (197.67 to 161.53, p=0.000; p<0.001) of heart rate was observed after introduction of IV salbutamol. Mean heart rate at the end of 12 hours of IV salbutamol was 113.20±9.05 (p=0.000; p<0.001). Neither cardiac arrhythmias nor elevated Troponin levels were observed in any of the patients. There was no significant hypokalaemia noted, the lowest serum potassium being 3.2meq/L.
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